关键词： Hemolytic uremic syndrome   Neurological involvement   Eculizumab   Magnetic resonance imaging  

摘要： BackgroundThrombotic microangiopathy (TMA) is a clinical syndrome encompassing a large group of rare but severe disorders including thrombotic thrombocytopenic purpura (TTP) and both typical and atypical forms of hemolytic uremic syndrome (HUS). The key role of the complement system is well known in TTP and atypical HUS, but recent reports describe its involvement in the pathogenesis of HUS secondary to gastrointestinal infections due to Shiga toxin-producing Escherichia coli (STEC).MethodsTMA mainly affects the kidney, but extra-renal complications are frequently described. The involvement of the central nervous system (CNS) represents often a life-threatening condition and it can result in serious long-term disability in HUS patients who overcome the acute phase of illness. In the present study, we retrospectively analyzed a pediatric cohort of a single tertiary pediatric hospital in Southern Italy, in which this complication occurred in 12/54 children (22% of cases), of whom five with severe neurological involvement had been successfully treated with *** great clinical variability of brain injury in our cohort has led us to retrospectively build a neurological score useful to assess the clinical severity of neurologic involvement. Subjects with higher neurologic score due to the most severe CNS involvement resulted in the group of patients early treated with eculizumab, obtaining a good clinical response (four out five patients). In conclusion, the early treatment with eculizumab in children with severe neurological involvement during STEC-HUS was associated with complete regression of both acute kidney injury (AKI) and neurological lesions observed at magnetic resonance imaging (MRI).ConclusionsA neurological score may be a useful tool to drive the early treatment of CNS complications in STEC-HUS with eculizumab, although future perspective controlled studies are urgently needed to validate this therapeutic approach.

关键词： Out-of-hospital cardiac arrest   Emergency medical services   Cardiopulmonary resuscitation   Epiemiology  

摘要： Aim: The impact of prehospital physician care for out-of-hospital cardiac arrest (OHCA) on long-term neurological outcome is unclear. We aimed to determine the association between emergency medical services (EMS) physician-led cardiopulmonary resuscitation (CPR) versus paramedic-led CPR and neurologically intact survival after OHCA. Methods: We assessed 613,251 patients using All-Japan Utstein Registry data from 2011 to 2015 retrospectively. The main outcome measure was 1-month neurologically intact survival after OHCA, defined as Cerebral Performance Category 1 or 2 (CPC 1-2). Results: Before propensity score matching, the 1-month CPC 1-2 rate was significantly higher in EMS physician-led CPR than in paramedic-led CPR [5.7% (1114/19,551) vs. 2.5% (14,859/593,700), P < 0.001;adjusted odds ratio (aOR), 1.50;95% confidence interval (CI), 1.40-1.61]. After propensity score matching, EMS physician-led CPR showed more favourable neurological outcomes than paramedic-led CPR [6.0% (996/16,612) vs. 4.6% (766/16,612), P < 0.001;aOR, 1.44;95% CI, 1.29-1.60]. In most subgroup analyses after matching, physician-led CPR had higher 1-month CPC 1-2 rates than paramedic-led CPR did;however, 1-month CPC 1-2 rates were similar between the two CPR configurations for patients aged < 18 years (5.6% vs. 8.2%, P = 0.10;aOR, 0.82;95% CI, 0.46-1.47) and those who received bystander defibrillation (26.3% vs. 21.5%;P = 0.10;aOR, 1.07;95% CI, 0.74-1.53). Conclusion: Within the limitations of this retrospective observational research, EMS physician-led CPR for OHCA was associated with improved 1-month neurologically intact survival compared with paramedic-led CPR. However, neurologically intact survival was similar for patients aged < 18 years and those receiving bystander defibrillation.

摘要： High levels of antibodies against glutamic acid decarboxylase (GAD) are observed in patients with different neurological disorders, but cells producing these autoantibodies are largely unexplored. We detect circulating GAD-reactive B cells in peripheral blood that readily differentiate into antibody-producing cells. These cells are highly elevated in most patients with GAD-antibody-associated disorders (n = 15) compared to controls (n = 19). They mainly produce GAD65 antibodies of the IgG1 and IgG4 subclasses and are as abundant as B cells reactive for common recall antigens. Bone marrow cells represent an additional source of GAD antibodies. The identification of GAD-antibody-producing cells has implications for the selection of cell-specific biologics. ANN NEUROL 2019;85:448-454.

关键词： Spine surgery   Intraoperative neuromonitoring   Sensitivity   Specificity   Complications  

摘要： PurposeWe compared the value of different uni- and multimodal intraoperative neurophysiological monitoring (IONM) methods on the detection of neurological complications during spine *** data derived from sensory spinal and cortical evoked potentials combined with continuous electromyography monitoring, motor evoked potentials and spinal recording were evaluated in relation to subsequent post-operative neurological changes. Patients were categorised based on their true-positive or true-negative post-operative neurological *** 2728 consecutive patients we had 909 (33.3%) IONM alerts. We had 8 false negatives (0.3%) with post-operative radicular deficit that completely recovered within 3months, except for one. There was no false negative for spinal cord injury. 107 were true positives, and 23 were false positives. Multimodal IONM sensitivity and specificity were 93.0% and 99.1%, respectively. The frequency of neurological complications including minor deficits was 4.2% (n=115), of which 0.37% (n=10) were permanent. Analysis of the single IONM modalities varied between 13 and 81% to detect neurological complications compared with 93% when using all *** IONM is more effective and accurate in assessing spinal cord and nerve root function during spine surgeries to reduce both neurological complications and false-negative findings compared to unimodal monitoring. We recommend multimodal IONM in all complex spine surgeries. [GRAPHICS] .

摘要： Orthotopic liver transplantation (LT) remains the only way to definitively cure patients with the most severe liver diseases. Because the survival rate is now fairly high, important questions about neurological sequelae or quality of life after LT have emerged. Indeed, LT represents a peculiar situation because up to 30% of patients present with neurological symptoms after LT compared with only 4% after cardiac transplant and 0.5% after renal transplant. These postoperative neurological symptoms have long been interpreted as sequelae of hepatic encephalopathy (HE). However, postoperative decompensation of an unknown cerebral condition due to the pathophysiology of cirrhosis or undiagnosed neurodegenerative disorders or aging constitute other possibilities that are underrecognized. Some patients who undergo LT for acute liver failure and patients with cirrhosis without episodes of HE and without any previous cerebral alteration also display post-LT neurological symptoms. This latter situation speaks in favor of a direct adverse effect of either general anesthesia, the surgical procedure, or factors related to the postoperative intensive care unit (ICU) environment. The role of inflammation, which has been described in the ICU setting, could also be a crucial determinant. In this review, we will discuss the neurological complications associated with LT, the neurocognitive complications after LT, and how to assess the LT-related neurological or neurocognitive complications. Furthermore, we will review the various hypotheses surrounding post-LT neurocognitive impairment and will conclude with recommendations for future directions.

关键词： THORACIC vertebrae   LUMBAR vertebrae   THORACIC vertebrae injuries   MAGNETIC resonance imaging   ORTHOPEDIC braces   VERTEBROPLASTY  

摘要： MRI after a CT scan for thoracolumbar spine (TLS) trauma has become commonplace because of the concerns for detection of posterior ligamentous complex injuries in the absence of substantial scientific evidence to support its use. We hypothesized that MRI scans were not necessary in the clinical management of TLS fractures. A prospective study was conducted at our Level I trauma center. A total of 39 neurologically intact patients with TLS fracture on CT were enrolled. The patients' CT scan and neurological examination were reviewed by a senior neurosurgeon, who determined clinical management based on these data. Assessment was repeated after an MRI of the spine was performed, and a second clinical plan was devised. The two treatment schemes were then compared. MRI resulted in a change in clinical management in 15 per cent of patients. Ten per cent of patients changed from requiring a brace to no brace and merely observation alone. In no patient planned for nonoperative care was surgery deemed necessary after completion of MRI. Among five patients with initial plans for operative intervention, two avoided surgery after the MRI. MRI has little impact on the management of patients with CT-proven thoracic and lumbar spine fractures. Only when surgery is planned based on CT studies does an MRI seem to assist with determining optimal care.

关键词： Cerebrum   active participation   Neurosciences   Rare Diseases   Awareness  

关键词： Balance beam test   Cerebral ischemia-reperfusion injury   Fault-foot test   MEG3   Neurological impairment   Neuronal apoptosis   Neuronal necrosis   Wnt/β-catenin signaling pathway  

摘要： Highlights • MEG3 was highly expressed in rats with cerebral IRI. • Repression of MEG3 promoted neurologic function restoration. • Repression of MEG3 alleviated rats with cerebral IRI from brain injury. • Inhibition of MEG3 promotes activation of wnt/β-catenin signaling pathway. • Inhibition of wnt/β-catenin signaling pathway reverses the down-regulation of MEG3 on brain nerve injury of MCAO rats. Abstract Objective This study was performed to investigate effect of long non-coding RNA (lncRNA) MEG3 on nerve growth and neurological impairment in a rat model after cerebral ischemia-reperfusion injury (IRI) through the Wnt/β-catenin signaling pathway. Methods Rat models of middle cerebral artery occlusion (MCAO) were established to stimulate an environment of cerebral IRI. The modeled rats were subjected to negative control (NC), MEG3, si-MEG3, classical Wnt pathway inhibitor DKK1 or classical Wnt pathway activator LiCl to validate the effect of MEG3 on neurological impairment and nerve growth. Neurological deficit scoring, fault-foot test and balance beam test were performed to assess neurological impairment. TTC staining, dry-wet weight method and Evan's blue (EB) staining were employed to determine infarct area, water content of brain tissues and blood-brain barrier (BBB) permeability, respectively. Neuronal apoptosis and necrosis were observed by TUNEL staining and Fluoro-Jade C staining. ELISA was adopted to identify levels of nerve growth factors to identify neurogenesis conditions, including brain derived neurotrophic factor (BDNF), nerve growth factor (NGF) and basic fibroblast growth factor (bFGF). Nissl staining was used to detect the survival of neurons in brain tissues of rats. Western blot analysis was used to detect the expression of key proteins in Wnt/β-catenin signaling pathway in brain tissues. Results High expression of MEG3 was identified in rat models of MACAO, the brain tissues of which manifested obvious neurological impairment, increased infarct

关键词： Gluten sensitivity   Coeliac disease   Ataxia   Neuropathy   Gluten-free diet   Neurological manifestations  

摘要： Purpose of reviewThe aim of this paper was to overview the current literature in order to establish the available treatment options for the neurological manifestations of gluten-related disorders (serologically confirmed gluten sensitivity and coeliac disease).Recent findingsA range of debilitating neurological manifestations is increasingly being recognized in patients with gluten sensitivity with and without enteropathy even in the absence of gastrointestinal symptoms. Ataxia is the commonest neurological manifestation, followed by peripheral neuropathy. Epilepsy, headache, encephalopathy, various movement disorders, cognitive impairment, and muscle disorders have also been linked to gluten sensitivity and coeliac disease and are discussed in this *** gluten-free diet is an effective first-line treatment of the neurological manifestations of gluten-related disorders. Very few patients will require additional immunosuppressive treatment usually in the form of mycophenolate.

关键词： adult   early-treated   neurological   newborn screening   phenylketonuria  

摘要： Newborn screening for phenylketonuria (PKU) and early introduction of dietary therapy has been remarkably successful in preventing the severe neurological features of PKU, including mental retardation and epilepsy. However, concerns remain that long-term outcome is still suboptimal, particularly in adult patients who are no longer on strict phenylalanine-restricted diets. With our systematic literature review we aimed to describe the neurological phenotype of adults with early-treated phenylketonuria (ETPKU). The literature search covered the period from 1 January 1990 up to 16 April 2018, using the NLM MEDLINE controlled vocabulary. Of the 643 records initially identified, 83 were included in the analysis. The most commonly reported neurological signs were tremor and hyperreflexia. The overall quality of life (QoL) of ETPKU adults was good or comparable to control populations, and there was no evidence for a significant incidence of psychiatric disease or social difficulties. Neuroimaging revealed that brain abnormalities are present in ETPKU adults, but their clinical significance remains unclear. Generally, intelligence quotient (IQ) appears normal but specific deficits in neuropsychological and social functioning were reported in early-treated adults compared with healthy individuals. However, accurately defining the prevalence of these deficits is complicated by the lack of standardized neuropsychological tests. Future research should employ standardized neurological, neuropsychological, and neuroimaging protocols, and consider other techniques such as advanced imaging analyses and the recently validated PKU-specific QoL questionnaire, to precisely define the nature of the impairments within the adult ETPKU population and how these relate to metabolic control throughout life.